Vision and Eye Issues in hEDS
Vision-related complications are common in individuals with hypermobile Ehlers-Danlos (hEDS) a disorder that affects collagen-rich ocular structures. These symptoms range from chronic discomfort to rare but serious events such as retinal detachment. Understanding the causes, symptoms, progression, and treatment options is key to managing ocular health in hEDS.
Causes: Why hEDS Affects the Eyes
hEDS involves abnormal collagen or extracellular matrix (ECM) function, which undermines the structural integrity of the sclera, cornea, lens, eyelids, and extraocular muscles. This fragility can lead to
Increased corneal curvature → contributes to myopia
Reduced tear production & eyelid laxity → causes dry eye (xerophthalmia)
Weakness in extraocular muscle support → leads to strabismus and diplopia
Scleral thinning → reveals underlying pigment, causing blue sclerae
Abnormal vitreoretinal interface → increases rare risk of retinal detachment
Lens support abnormalities → minor lens opacities or curvature changes
Eyelid laxity → floppy eyelid syndrome, exposure-related irritation
Prevalence
Vision problems are common in people with hEDS, especially dry eye, high myopia, and convergence insufficiency. In one adult study, 100% of participants had dry eye symptoms, 15.9% had pathologic myopia, and 13.6% had minor lens opacities. Serious complications like retinal detachment and keratoconus are rare. Children and older adults are also affected, but detailed prevalence data for these age groups are limited.
Common Ocular Manifestations in hEDS
Condition | Cause | Symptoms | Progression | Treatment |
Dry Eyes (Xerophthalmia) | Reduced tear production, lid laxity | Grittiness, photophobia, blurred vision | Chronic | Artificial tears, punctal plugs, lid care |
Myopia (often high/pathologic) | Elongated globe, steep cornea | Blurred distance vision, eye strain | May worsen in adolescence | Glasses, contacts (avoid LASIK in hEDS) |
Strabismus (incl. convergence insuff.) | Muscle imbalance or ligament laxity | Double vision, poor focus, fatigue | May develop over time | Prism lenses, vision therapy, cautious surgery |
Blue Sclerae | Scleral thinning | Usually asymptomatic, visible blue tint | Stable | None |
Retinal Detachment (rare) | Vitreoretinal interface weakness | Floaters, flashes, vision loss | Acute emergency | Surgical repair (prompt intervention) |
Steep Corneas | Collagen instability | Can affect vision, linked to myopia | May lead to keratoconus | Scleral lenses, monitoring |
Minor Lens Opacities | Weak zonular fibers or early lens changes | Glare, blurred vision in later stages | May progress with age | Glasses, eventual surgery (with caution) |
Eyelid Laxity / Floppy Eyelid Syndrome | Weak tarsal plate and skin tissue | Irritation, exposure during sleep | Chronic | Eye shields, lubricants, surgery (rare) |
Abnormal Vitreous Structure | Collagen irregularities in vitreous body | Floaters, rarely detachment | Variable | Monitor for retinal changes |
Symptoms
Common visual and eye-related symptoms in hEDS include
Blurred or fluctuating vision
Dryness, irritation, or foreign body sensation
Photophobia (light sensitivity)
Diplopia (double vision) or difficulty focusing
Eye fatigue or strain
Floaters or flashes (potential warning sign for retinal detachment)
Eyelid irritation or exposure during sleep
Progression and Risk
Dry eye and refractive errors are typically chronic and may worsen with age.
Strabismus and convergence insufficiency may emerge gradually and are more common in children and adults.
Minor lens opacities may progress slowly in older adults but rarely cause severe impairment.
Retinal detachment is rare but serious—requires emergency care.
Pathologic myopia may be more common in hEDS than the general population, especially during adolescence.
Age-Specific Considerations and Prevalence
Children
Common: dry eyes, myopia (nearsightedness), and convergence insufficiency ((an eye coordination problem).
May also have subtle eyelid laxity or periocular features (visible characteristics or changes in the area around the eyes e.g., creases).
Serious complications like keratoconus (the cornea becomes cone-shaped, causing distorted vision) or retinal detachment are rare.
Vision symptoms can impact school performance and cause fatigue or discomfort during reading or screen use.
Adults
Dry eye is the most frequently reported issue.
About 15.9% may have pathologic myopia (a severe form of nearsightedness); 13.6% may have minor lens opacities.
Convergence insufficiency (an eye coordination problem) and eye fatigue are common.
Steep corneas and increased lens curvature may appear but don’t always require treatment.
Quality of life may be affected by cumulative symptoms such as irritation, blurred vision, or sensitivity to light.
Older Adults
Minor lens opacities tend to increase with age.
Tissue fragility and medication side effects (e.g., antihistamines) may worsen dry eye.
No strong evidence for increased risk of cataract, glaucoma, or macular degeneration beyond typical aging.
Surgical planning (e.g., cataracts) should take healing complications into account.
Management and Treatment Guidelines
Routine Monitoring
Annual eye exams are recommended.
Watch closely for:
Worsening dry eye
Refractive changes
Double vision or eye alignment issues
Progressive lens opacity or floaters
Dry Eye Treatment
Artificial tears (preservative-free)
Punctal plugs to retain tears (tiny, biocompatible devices that are inserted into the tear ducts to relieve dry eyes)
Lid hygiene and warm compresses
Environmental adjustments (e.g., humidifiers)
Omega-3 fatty acids may help some patients
Refractive Error Treatment
Glasses and soft or scleral contact lenses
Avoid LASIK and corneal surgeries due to risk of corneal instability
Monitor for changes in myopia, especially during adolescence
Strabismus (Eye Misalignment) and Diplopia (Double Vision)
Prism lenses for mild misalignment
Vision therapy for convergence insufficiency
Eye muscle surgery only when necessary—requires extra caution
Surgical Precautions
Eye surgeries (cataract, strabismus, retinal) should be done with awareness of tissue fragility
Risks include poor healing, infection, or worsening symptoms
Pre-surgical consultation should include hEDS-related risks
Emerging and Theoretical Notes
Microstructural changes in the cornea may contribute to symptoms but are not fully understood.
Blue sclerae are more common in other EDS types but may appear in hEDS.
Retinal arterial tortuosity has been observed in a small number of hEDS patients but its relevance is not yet clear.
Mast cell–related inflammation may contribute to dry eye or allergic symptoms in some individuals.
Summary
Hypermobile Ehlers-Danlos syndrome affects several parts of the eye due to fragile connective tissue, leading to symptoms such as dry eye, myopia, lens opacities, and occasional eye misalignment. These symptoms can vary with age, with dry eye and myopia being common across the lifespan, and convergence insufficiency more frequent in children and adults. One study found dry eye symptoms in all participants with hEDS, with 15.9% showing pathologic myopia and 13.6% showing lens opacities. Although rare, complications like retinal detachment can occur and require emergency care. Surgical treatment may be more risky due to tissue fragility, and standard ophthalmic care should be tailored to this population. With regular monitoring and appropriate treatment, most people with hEDS can manage their vision well and avoid serious complications.
